Pulmonary Vasodilator Use in Children with Heterotaxy Syndrome Undergoing Surgical Intervention for Congenital Heart Disease

Abstract

Purpose Patients with heterotaxy syndrome have a high rate of congenital heart disease, often requiring surgical interventions, including palliative procedures to augment or restrict pulmonary blood flow. Pulmonary hypertension in this setting complicates surgical interventions and overall management. Pulmonary vasodilators may be used to treat pulmonary hypertension and facilitate surgical interventions. We describe the use of pulmonary vasodilators in patients with heterotaxy syndrome. Methods Single-center retrospective chart review of patients with heterotaxy syndrome who underwent at least one cardiac operation from Jan 1 2008 to Oct 31 2019. The diagnosis of heterotaxy syndrome was established from imaging studies performed prior to intervention. Surgical interventions were reviewed from operative reports. Pulmonary vasodilator use was assessed from progress notes and medication reconciliation at all inpatient and outpatient encounters. Results Thirty-four patients with heterotaxy syndrome were treated at our center during the study period, with follow up ranging from 18 to 3385 days. Palliative surgical interventions included restriction of pulmonary blood flow in 8 patients and augmentation of pulmonary blood flow in 11 patients. Pulmonary vasodilators were used in 16 patients (47%): 7 (20%) received inhaled nitric oxide while admitted, 3 (9%) received supplemental oxygen as outpatients, 12 (35%) received sildenafil as outpatients, 5 (14%) received multiple agents. Treatment was initiated in the inpatient setting in all patients. All patients on supplemental oxygen and the majority of patients on sildenafil continued on treatment at most recent follow up (10 patients, 83% of patients ever prescribed). There was no difference in pulmonary vasodilator use when stratified by surgical palliation strategy, and no differences in survival between patients with and without pulmonary vasodilator use. Conclusion Pulmonary vasodilator use is common in patients with heterotaxy syndrome undergoing surgical interventions for congenital heart disease. It is typically initiated in the inpatient setting but well-tolerated on a long-term basis, and may facilitate treatment in this challenging population.